Thirty-four patients with connective tissue diseases used dexamethasone drops 01% in one eye for 6 weeks. There was a higher incidence of positive steroid response than would be expected in a normal population. Most of the male patients were responders. Care should be exercised when prescribing local steroids for these patients. Males should be offered regular screening for glaucoma. Eye involvement is a prominent feature of connective tissue diseases (CTD). Keratoconjunctivitis sicca (KCS) is the commonest complication, occurring in 11% of patients with rheumatoid arthritis (RA)I and sporadically in systemic lupus erythematous (SLE), progressive systemic sclerosis (PSS), polymyositis, and psoriatic arthritis. Scleritis appears in 067% of RA patients and in other cases of CID (e.g., ankylosing spondylitis and Behget's disease) from time to time. Uveitis is of particular importance in juvenile rheumatoid arthritis, ankylosing spondylitis, and Reiter's syndrome. Conjunctivitis is one of the diagnostic criteria for Reiter's syndrome. Proptosis, diplopia due to extraocular myopathy, corneal disturbances, optic neuritis, and retinal vascular changes may occur is SLE and lid shortening and iris atrophy in PSS. Discolouration and oedema of the lids and extraocular muscle changes occur in polymyositis and dermatomyositis and retinal vasculitis in up to 20% of patients with polyarteritis nodosa. The large host of ocular phenomena that are reported make it unlikely that the trabecular meshwork is exempt from autoimmune inflammatory processes, though glaucoma is not a recognised complication of CTD. The intertrabecular spaces of the 'pore' area of the trabecular meshwork are lined by a layer of mucopolysaccharide.2 If this layer is removed by hyaluronidase, resistance to aqueous outflow falls. Topical steroids increase outflow resistance. This may be due to increased production of mucopolysaccharide

KERATO-CONJUNCTIVITIS sicca is the condition which follows a reduction of secretion by the main and accessory lacrimal glands, and is characterized by interpalpebral staining of the bulbar conjunctiva with rose Bengal, punctate corneal staining with punctate opacities in Bowman's zone, the presence of thick stringy mucous strands in the lower fornices, and corneal filaments, the latter being neither invariable nor pathognomonic. Kerato-conjunctivitis sicca occurs most commonly as a manifestation of Sjogren's syndrome; it is occasionally seen in sarcoidosis due to infiltration of the lacrimal gland, and is rarely due to congenital absence of tears (e.g. the Riley-Day syndrome) or acquired neurogenic lesions of the secretomotor pathways. Sjogren (1933) described the triad of clinical features (rheumatoid arthritis, xerostomia, and kerato-conjunctivitis sicca) and stated that two of the three should be present. It is now recognized that any of the collagen diseases, but particularly disseminated lupus erythematosus (DLE), may replace rheumatoid arthritis in the triad, and that Sjogren's syndrome forms part of the range of connective tissue disorders associated with the presence of autoantibodies (Morgan and Castleman, 1953; Bain, 1960; Robinson, 1963). The frequent poor response to treatment of this disorder is indicated by the variety of methods which have been used, including various artificial tear preparations, local steroids, anticoagulants (Stark, 1961), fibrinolysin (Weve, 1928), debridement of the epithelium, radiation (Winters and Asbury, 1956), systemic hydroxychloroquine (Heaton, 1963), tarsorrhaphy, and contact lenses, while occlusion of the puncta certainly helps to conserve any lacrimal secretion which may be formed. Duke-Elder and Leigh (1965) have concluded that "in the majority of such cases the ophthalmologist is reduced to the expedient of judicious but impotent expectancy". The best available artificial tear preparation appears to be a solution of carboxymethylcellulose at pH 8 5, with added sodium bicarbonate and sodium chloride as devised by Jones and Coop (1965), and this is the standard tear substitute used here. Jones and Coop also reported encouraging results in fifteen patients with keratoconjunctivitis sicca treated with the mucolytic agent acetylcysteine. N-acetyl-L-cysteine is a derivative of the amino-acid L-cysteine and is widely used to reduce the viscosity of mucus in a variety of broncho-pulmonary disorders (Webb, 1962). This reduction in viscosity is achieved by the reducing action of the free sulphydryl group in the molecule on the disulphide bonds of the mucoproteins present in mucus.

SYNOPSIS A case of myotubular myopathy is described which is unusual because of bilateral cataracts and prominent myotonic-like discharges on the EMG. The significance of these findings is discussed in relation to dystrophia myotonica.Myotubular myopathy is a slowly progressive muscular disorder starting in childhood, affecting both skeletal and extraocular musculature. Spiro et al. (1966) employed the word 'myotubular' because of the resemblance of the myofibrils to fetal myotubules, and suggested that the disorder might be due to arrested maturation of the developing muscle. This suggestion has been disputed (Sher et al., 1967) and the more neutral term of 'centronuclear myopathy' is preferred by them. There are now more than 20 reports of the disorder but none mentions the presence of cataract and very few have discovered myotonic discharges on the EMG. The present case had both cataracts and electrical myotonia which initially raised the question of dystrophia myotonica until the muscle was examined histologically. CASE REPORTThe patient was first seen by us at the age of 29 years, when he was admitted to the Wessex Neurological Centre for assessment of muscular weakness. Hitherto, he had been assumed to suffer from dystrophia myotonica but there were several inconsistent features.At the age of 2 years he was noted to have a contracture of both Achilles tendons and at 4 years displayed torticollis as a result of contracture of the right sternomastoid muscle. When 10 years old he was found to have bilateral ptosis, severe ophthalmoplegia, diffuse muscular weakness, and wasting involving the face, limb girdles, and limbs, but no

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Four cases of presumed ocular histoplasmosis like retinopathy are presented. A detailed immunological assessment was carried out on the patients and a control group: lymphocyte immunophenotyping; flow cytometric analysis; HLA typing and T cell receptor variable region (TCR V region) expression were assessed. Analysis of TCR V region expression revealed no significant preferential expression. HLA typing also failed to reveal any links. All lymphocyte markers analysed were unremarkable, with the exception of CD38 which was significantly raised compared with controls (p < 0.01). This finding was confirmed by the use of two different CD38-specific monoclonal antibodies. The raised CD38 in our cases was shown to be persistent when the patients were retested after an interval of several months. Significantly, this may correlate with poor T cell function, as in Common Variable Immunodeficiency, making these patients more susceptible to various stimuli.